11/27/2007 Case 2: Subendothelial vacuolization in choroid plexus

Clinical History:

A 25-day-old full-term female born to a 28-year-old woman presented
after prolonged rupture of membranes and positive maternal culture for
group B Streptococcus. The infant was floppy at birth with APGAR
scores of 5, 6, and 9 at 1, 5, and 10 minutes, respectively. Workup
was started in the NICU and ampicillin/gentamicin was started.
Echocardiogram showed cardiomegaly with cardiac dysfunction and she was
started on dobutamine, milrinone drip and Lasix. Repeat sepsis
evaluation was done and antibiotics were changed to
vancomycin/ampicillin and meropenem. TORCH work up, metabolic
evaluation and karyotype were normal. At birth, blood pressure was
51/34. By day 5 of life, it ranged from 96-105/60. She was put on
sodium nitroprusside, esmolol, labetalol drip, milrinone drip, Lasix
and hydralazine. She failed to respond and phenoxybenzamine was added.
She was on Actigall for hyperbilirubinemia. On abdominal sonogram, a
complex, cystic mass was seen in the right adrenal. This was
interpreted as an evolving adrenal hemorrhage. CT scan of the abdomen
confirmed this finding and showed possible vascular compromise to the
right kidney. Pertinent lab data: dopamine 1520 pg/mL, epinephrine
316 pg/mL, norepinephrine 4484 pg/mL, plasma renin activity 121.67
ng/mL/hr. Plasma metanephrine was elevated. Significant physical exam
findings included: severe jaundice, distended abdomen with
hepatomegaly. A hard palpable mass on the anterior axillary margin of
the left arm was suspicious for metastatic neuroblastoma. No
neuroblastoma was present in the biopsy. The infant became hypoxic and
bradycardic. She developed asystole and died on her twenty-fifth day
of life.

Diagnostic Notes:

The e-mailed photomicrograph showed choroid plexus of the autopsy
brain. Subendothelial vascular wall cells, only in choroid plexus and
not in vessels of other tissues, were large and vacuolated. Other
brain findings were hypoxic including in the pons and subiculum as well
as in the, hippocampal CA-1 region. The question arose as to the nature
of the subendothelial foamy cells in the vessels in the choroid plexus
stroma. Choroid plexus epithelium is well preserved, so artifact was
questionable but not ruled out. Whipple's disease had also been
considered. Other stains were considered by the group but based on the
current findings of subendothelial foamy cells in the vessels in the
choroid plexus stroma no specific diagnosis was offered.